Blood Disorders (Exam II) Marcus's Cards Flashcards

1
Q

What are the S/S of vWF disorder?

A

Easy bruising
epistaxis
menorrhagia

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2
Q

Von Willabrand Factor Disorder facts

A

-vWF critical role in platelet adherence/adhesion
-Most common hereditary bleeding disorders

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3
Q

Classifications of Inherited vonWillebrand Disease

A

Type I: Partial quantitative deficiency of vWF
mildest; most common; responds to DDAVP
Type IIA and IIM: Dysfunction of platelet adhesion
May respond to DDAVP
Type IIB: Increased platelet binding affinity
Thrombocytopenia with DDAVP
Type IIN: Decreased F VIII-binding affinity
often confused with hemophilia A
Type III: Severe quantitative deficiency of vWF
Rarest; most severe; usually requires factor concentrates

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4
Q

What would lab values be for someone with vWF deficiency?

A
  • Normal PT & aPTT
  • Bleeding time is prolonged
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5
Q

What are the treatments for vWF deficiency?

A
  • Desmopressin 1st line
  • Cryoprecipitate
  • Factor VIII (or transfusion of specific factor)
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6
Q

How does DDAVP work in regards to treatment of von Willebrand deficiency??

A

Stimulates vWF release from endothelial cells (it’s a synthetic analogue of vasopressin)

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7
Q

What is the dose for DDAVP?

A

0.3 mcg/kg in 50 mL over 15-20 mins (Do not bolus)

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8
Q

What is the Peak & duration of DDAVP?

A
  • Peak: 30mins
  • Duration: 6-8hrs
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9
Q

What are side effects of DDAVP?

A
  • HA
  • Rubor
  • hypotension
  • tachycardia
  • hyponatremia
  • water intoxication (excessive water retention)
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10
Q

What is the most major side effect of DDAVP?

A

Hyponatremia

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11
Q

Someone that gets DDAVP needs to be on what?

A

Fluid restriction 4-6hrs before & after DDAVP

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12
Q

What CNS and EKG changes will you see with serum Na of 120 meq/L

A

CNS: Confusion and restlessness
EKG: maybe widening of QRS

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13
Q

What CNS and EKG changes will you see with serum Na of 115 meq/L

A

CNS: Somnolence and nausea
EKG: elevated ST segments and widened QRS

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14
Q

What CNS and EKG changes will you see with serum Na of 110 meq/L

A

CNS: seizures, coma
EKG: Vtach or Vfib

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15
Q

What blood product can be utilized for vWF disease if the patient is unresponsive to DDAVP?

A

Cryoprecipitate

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16
Q

1 unit of Cryo raises the ____ level by ___?

A

Fibrinogen by 50 mg/dL

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17
Q

What is a potential risk factor with cryoprecipitate?

A
  • Increased risk of infection (not submitted to viral attenuation)
  • Multiple donors
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18
Q

How is factor VIII made?

A
  • Pool of plasma from a large number of donors.
  • it does undergo viral attenuation
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19
Q

What does factor VIII concentrate contain?

A

Contains Factor VIII and vWF

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20
Q

When is Factor VIII given?

A

Preop or intraop

21
Q

When should DDAVP be given prior to surgery?

A

60mins before Sx

22
Q

Anesthesia Consideration for vWF disease

A
  • Prior evaluation by a hematologist
  • Normalization of the bleeding time and improved levels of F VIII should be confirmed before the surgery in patients
  • General anesthesia is preferred
  • Patients with coagulopathies undergoing neuroaxial block will have increased risk of developing a hematoma and compression of neurological structures
23
Q

What things to avoid in giving anesthesia to a patient with vWF disease?

A
  • Avoid trauma in anesthesia
  • A-lines aren’t recommended
  • Laryngeal trauma during intubation may cause a hematoma
  • IM medication is avoided
24
Q

What blood product poses an increase risk for infection? Why?

A
  • Cryoprecipitate
  • Not sent for viral attenuation
25
Q

Pts with coagulopathies undergoing neuraxial anesthesia are at increased risk for what?

A
  • Hematoma
  • Nerve compression
26
Q

How does heparin work?

A
  • Thrombin inhibition: which prevents the conversion of fibrinogen to fibrin
  • Antithrombin III activation
27
Q

What labs are monitored with heparin? and how can we reverse it?

A
  • PTT &/or ACT
  • reversible by protamine: a positively charged polypeptide forming a stable complex neutralizing heparin
28
Q

What is the mechanism of action of Coumadin?

A

Inhibition of vitamin K-dependent factors: II, VII, IX, X

29
Q

What is the onset for Vitamin K administration?

A

6-8hrs

30
Q

What drugs/products can be given to reverse coumadin faster than Vit K?

A
  • Prothrombin complex concentrates
  • Factor VIIa
  • FFP
31
Q

What is the mechanism of action for fibrinolytics (Urokinase, streptokinase & tPA)?

A

Convert plasminogen to plasmin, which cleaves fibrin

32
Q

How do tranexamic acid (TXA), aminocaproic acid, and aprotinin work?

A

Inhibit conversion of plasminogen to plasmin

33
Q

Treating patients who are on antiplatelet therapy

A
  • D/C drugs on time
  • platelet transfusion (if you have to operate emergently) becuase these medicaion irrversibly bind to the platelet
34
Q

What is disseminated intravascular coagulopathy?

A
  • Systemic activation of the coagulation system simultaneously leads to thrombus formation and exhaustion of platelets and coagulation factors
  • Numerous underlying disorders may precipitate DIC, including trauma, amniotic fluid embolus, malignancy, sepsis (longer onset except for urosepsis), or incompatible blood transfusions
35
Q

What is the best way to treat DIC?

A

Treat the underlying cause

36
Q

What will labs show for someone in DIC?

A
  • ↓Platelet count
  • Prolonged PT, PTT & TT.
  • ↑ fibrin degradation products
37
Q

When is antifibrinolytic therapy given to someone in DIC?

A

Trick question, it shouldn’t. Can lead to catastrophic thrombotic complications

38
Q

What should the treatment for DIC include?

A

Treatment includes blood component transfusions to replete coagulation factors and platelets consumed in the process

39
Q

What is factor V?

A
  • Protein for clotting.

Activated protein C inactivates factor V thus stopping clot growth.

40
Q

What is Factor V Leiden?

A

Genetic mutation where Activated protein C cannot stop factor V Leiden thus excessive fibrin.

41
Q

What does Activated Protein C do?

A

Inactivates factor V when enough fibrin has been made.

42
Q

Who is usually tested for Factor V Leiden?

A

Pregnant women. Especially ones with unexplained late stage abortions

43
Q

What anticoagulant medications could someone with Factor V Leiden be put on?

A
  • Warfarin
  • LMWH & unfractionated heparin
44
Q

Anesthesia implications of Factor V Leiden

A
  • Patients will be on anticoagulants becuase they have an increased risk of DVT or PE
45
Q

Pathology of HIT

A

HIT describes an autoimmune-mediated drug reaction occurring in as many as 5% of patients after exposure to unfractionated heparin or (rare cases) LMWH

Thrombocytopenia occurs 5-14 days after initial therapy

46
Q

What is the hallmark sign of HIT?

A

Plt count <100,000

thrombocytopenia

47
Q

HIT results in ____ activation and potential____?

A

platelet; thrombosis

48
Q

What is heparin replaced with when HIT is diagnosed?

A

Agratroban, lepirudin, or bivalirudin (direct-thrombin inhibitors)

49
Q

What is Fondaparinux & when is it used?

A
  • A synthetic Factor Xa inhibitor
  • used to treat VTE in HIT (off label use)
  • If possible, avoid future exposure to unfractionated heparin